Cystic fibrosis associated liver disease in children

Table 5 Indications and contraindications for liver transplantation in cystic fibrosis liver disease (Modified from Freeman et al[43])

Indications and contraindications
Indications
Strong	(1) Progressive hepatic dysfunction with hypoalbuminemia and coagulopathy (Coagulopathy not corrected by vitamin K, cholestasis not attributed to other causes); (2) Complications of portal hypertension (Intractable/recurrent variceal bleeding which is not controlled by medical or endoscopic management); (3) Hepatopulmonary and porto-pulmonary syndrome; (4) Overt hepatic encephalopathy; and (5) Hepatorenal syndrome
Controversial	(1) Deteriorating pulmonary function (FEV1/FVC <50%) with increased frequency and severity of pulmonary infective episodes requiring hospitalization; and (2) Severe malnutrition, unresponsive to intensive nutritional support
Contraindications
Absolute	(1) Extrahepatic malignancies not amenable to curative therapy; (2) Multiorgan disease for which transplant would not be considered life-sustaining; (3) Uncontrolled systemic or pulmonary infection, active exacerbation, or veno-arterial extracorporeal membrane oxygenation; and (4) Severe porto-pulmonary hypertension nonresponsive to medical management
Relative	(1) Hepatocellular carcinoma; (2) Noncompliance or psychosocial concerns unamenable to transplant; (3) Uncontrollable CF-related diabetes; (4) Substance abuse; (5) Severe cardiopulmonary disease; and (6) Infection/colonization with multi-resistant organism (e.g., Burkholderia cenocepacia and Mycobacterium abscessus)

FEV1: Forced expiratory volume in one second; FVC: Forced vital capacity.